Acquired Fanconi Syndrome with Proximal Tubular Cytoplasmic Fibrillary Inclusions of λ Light Chain Restriction
スポンサーリンク
概要
- 論文の詳細を見る
Light chain proximal tubulopathy is a rarely reported entity associated with plasma cell dyscrasia that classically manifests as acquired Fanconi syndrome and is characterized by the presence of κ-restricted crystals in the proximal tubular cytoplasm. We herein present a case of multiple myeloma with Fanconi syndrome and acute kidney injury due to light chain proximal tubulopathy with light chain cast nephropathy. Prominent phagolysosomes and numerous irregularly shaped inclusions with a fibrillary matrix in the cytoplasm of the proximal tubules were identified on electron microscopy. A monotypic light chain of the λ type was detected in the distal tubular casts, proximal tubular cytoplasmic lysosomes and fibrillary inclusions on immunofluorescence and immune electron microscopy. This case underscores the importance of conducting careful ultrastructural investigations and immunocytologic examinations of light chains for detecting and diagnosing light chain proximal tubulopathy.
- 一般社団法人 日本内科学会の論文
一般社団法人 日本内科学会 | 論文
- A Patient with Diffuse Cutaneous Systemic Sclerosis Complicated by Antineutrophil-cytoplasmic Antibody-associated Vasculitis Exhibiting Honeycomb Lung without Volume Loss
- Parkinson's Disease and the Cardio-ankle Vascular Stiffness Index
- Pseudohypoparathyroidism Type II in a Woman with a History of Thyroid Surgery
- A Novel Frameshift Mutation in Exon 4 Causing a Deficiency of High-molecular-weight Kininogen in a Patient with Splenic Infarction
- Risk Factors for Liver Injury with an Elevated Serum Bilirubin Concentration Caused by Antituberculous Drugs